Conformational behavior and aggregation of ataxin-3 in SDS. [PDF]
PLoS ONE, 2013 Spinocerebellar ataxia type 3 (SCA3) is one of nine polyglutamine (polyQ) diseases all characterized by the presence of intraneuronal inclusions that contain aggregated protein.
Helen M Saunders +3 more
doaj +7 more sources
Identification and functional dissection of localization signals within ataxin-3
Neurobiology of Disease, 2009 Spinocerebellar ataxia type 3 (SCA3) or Machado–Joseph disease (MJD) belongs to a group of autosomal dominant neurodegenerative diseases, which are caused by the expansion of a polyglutamine repeat in the affected protein, in this case ataxin-3. Ataxin-3
Paul Michel Aloyse Antony +6 more
doaj +6 more sources
NEDD8: A new ataxin-3 interactor [PDF]
Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, 2007 Machado–Joseph disease (MJD/SCA3) is an autosomal dominant neurodegenerative disease caused by the expansion of a CAG tract in the coding portion of the ATXN3 gene.
Rodrigues, Ana-João +14 more
core +5 more sources
Gene expression profiling in ataxin-3 expressing cell lines reveals distinct effects of normal and mutant ataxin-3 [PDF]
Journal of Neuropathology & Experimental Neurology, 2003 Spinocerebellar ataxia type 3 (SCA3) is a late-onset neurodegenerative disorder caused by the expansion of a polyglutamine tract Within the gene product, ataxin-3 We have previously shown that mutant ataxin-3 causes upregulation of inflammatory genes in ...
Vogt, IR +7 more
core +5 more sources
Valosin-containing protein (VCP/p97) is an activator of wild-type ataxin-3. [PDF]
PLoS ONE, 2012 Alterations in the ubiquitin-proteasome system (UPS) have been reported in several neurodegenerative disorders characterized by protein misfolding and aggregation, including the polylgutamine diseases.
Mário N Laço +4 more
doaj +2 more sources
Cerebellar soluble mutant ataxin-3 level decreases during disease progression in Spinocerebellar Ataxia Type 3 mice. [PDF]
PLoS ONE, 2013 Spinocerebellar Ataxia Type 3 (SCA3), also known as Machado-Joseph disease, is an autosomal dominantly inherited neurodegenerative disease caused by an expanded polyglutamine stretch in the ataxin-3 protein.
Huu Phuc Nguyen +5 more
doaj +2 more sources
Heterogeneous Intracellular Localization and Expression of Ataxin-3
Neurobiology of Disease, 1998 Spinocerebellar ataxia type 3 or Machado–Joseph disease (SCA3/MJD) is an autosomal dominant neurodegenerative disorder caused by an unstable and expanded CAG trinucleotide repeat that leads to the expansion of a polyglutamine tract in a protein of ...
Yvon Trottier +7 more
doaj +3 more sources
Nucleocytoplasmic shuttling activity of ataxin-3. [PDF]
PLoS ONE, 2009 Spinocerebellar ataxia type-3, also known as Machado-Joseph Disease (MJD), is one of many inherited neurodegenerative disorders caused by polyglutamine-encoding CAG repeat expansions in otherwise unrelated genes.
Sandra Macedo-Ribeiro +3 more
doaj +5 more sources
Allosteric Modulation of Pathological Ataxin‐3 Aggregation: A Path to Spinocerebellar Ataxia Type‐3 Therapies [PDF]
Advanced ScienceSpinocerebellar ataxia type 3 (SCA3) is a rare neurodegenerative disorder caused by the expansion of a polyglutamine (polyQ) repeat in ataxin‐3 (Atx3) for which no disease‐modifying therapies are available.
Alexandra Silva +28 more
doaj +2 more sources
Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties. [PDF]
PLoS ONE, 2010 Protein context clearly influences neurotoxicity in polyglutamine diseases, but the contribution of alternative splicing to this phenomenon has rarely been investigated.
Ginny Marie Harris +4 more
doaj +2 more sources